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Monday, October 3, 2011

Bad Blood

In 1817 the death of Princess Charlotte Augusta of Wales, George IV’s only legitimate child and George III’s only grandchild-heir sent the British monarchy into a spin. George IV’s three younger brothers suddenly realised that they would be tasked with the responsibility of preserving the lineage.

While George IV had twelve other healthy children all were illegitimate.

Alexadrina Victoria was born on the 24th May 1819, the first and only child of Prince Edward, Duke of Kent and Princess Victoria of Saxe-Coburg-Saalfeld. At the time of her birth she was fifth in line to the throne after her three uncles and father. Her father, aged 51 at the time of her birth, died a little over six months later. (3)

It’s possible that that his late-life decision to reproduce resulted in a genetic heritage that continues to haunt the royal houses of Europe today.

Victoria inherited the throne in 1838 after the death of her Uncle William IV. By all accounts she had an extremely happy life. Unlike many of he relatives she enjoyed a long and happy marriage. She gave birth to nine children who all, again unlike many of their relatives, made it into a healthy adulthood. They in turn produced forty grandchildren who were married throughout the royal houses of Europe. (3)

Victoria’s second to last child, Leopold, was known to be fragile. Small cuts and bumps would cause a great deal of bleeding and pain. Victoria suffered a great deal of anxiety about the health of her son and coddled him, much to his apparent distaste. Nontheless, Leopold survived to marry and produce two children, before dying as the result of an intracerebral haemorrhage following a fall. Taken by itself this may have meant nothing. However a disturbingly similar trend was beginning to be seen amongst Victoria’s male descendents.

The Queen allegedly found the appearance of the disease in her family upsetting and confusing and would protest that it did not originate from her side of the family. Given the understanding at the time this was not an unreasonable claim as no prior relative of Victoria was known to exhibit the disease. A whisper started about the ‘curse of the Coburgs’. The family of Prince Albert Saxe-Coburg, it was said, had been cursed by a Hungarian monk envious of their wealth. (2)

With the benefit of modern knowledge, however, the ‘blame’ can be placed squarely at the feet of the queen.

The ‘curse of the Coburgs’ is today known as haemophilia.

Haemophilia is a disease that affects a clotting factor in the blood rendering it ineffective. This clotting factor helps in the formation of long term clots. Therefore, while a haemophiliac does not bleed more profusely than an unaffected person they do bleed longer. Bleeding into joints and muscle is also much more common (4). In genetic studies haemophilia forms the classic example of a sex-linked recessive disorder with the mutation carried on the X chromosome (5).

What makes a human baby become male or female is determined in a very simple way. Two X chromosomes and you become a female. One X chromosome and one Y chromosome and you become a male.

If you’re female and carrying a haemophilic X chromosome the extra copy will compensate and you will be unaffected by disease.

If you are male you aren’t so lucky.

Haemophilia is exclusively a disease of men. However it is a disease exclusively carried by women. Queen Victoria was one such woman. As the disease was previously unseen in her family tree it seems very likely that the mutation that caused it also originated in her. Through her descendents haemophila spread to the royal houses of Prussia, Spain and Russia. A swathe of morbidity and mortality swept across Europe. In Russia the rise of Rasputin and the fall of the Russian royals in many ways related to the poor health of Prince Alexi who was stricken with the disease. (1)

Ironically, due to their inheritance through an unaffected male, the British Royal Family were spared. Victoria, fiercely proud of the country she ruled for 63 years, may have drawn some comfort from that.

1 comment:

This is utterly fascinating, as always. Now I know why there are so many diseases carried by women by only inherited by men. I always knew there was an advantage to having two proper chromosones, rather than those knock-off Ys.